Signs in neuroradiology - part 1

*



Fabrcio Guimares Gonalves
I
; Filipe Ramos Barra
II
; Valter de Lima Matos
III
;
Cssio Lemos Jovem
II
; Lzaro Lus Faria do Amaral
IV
; Raquel delCarpio-
O'Donovan
V

I
Titular Member of Colgio Brasileiro de Radiologia e Diagnstico por Imagem (CBR), Clinical
Fellow in Neuroradiology at the Montreal General Hospital, McGill University Health Centre
(MUHC), Montreal, Quebec, Canada
II
MDs, Residents in Radiology and Imaging Diagnosis at the Hospital Universitrio de
Braslia, Braslia, DF, Brazil
III
MD, Neuroradiologist at the Hospital Universitrio de Braslia and Hospital Santa Luzia,
Braslia, DF, Brazil
IV
MD, Neuroradiologist, Head of the Department of Neuroradiology at Medimagem - Hospital
da Beneficncia Portuguesa de So Paulo and Hospital Santa Catarina, So Paulo, SP, Brazil
V
MD, Neuroradiologist, Professor of Radiology, Director of the Neuroradiology Fellowship
Program, McGill University Health Centre (MUHC), Montreal, Quebec, Canada
Mailing Address



ABSTRACT
The use of signs or analogies for interpretation and description of medical images is an old
and common practice among radiologists. Comparison of findings with animals, food or
objects is not unprecedented and routinely performed. Many signs are quite specific and, in
some cases, pathognomonic. Indeed, notwithstanding their degree of specificity, signs may
help in the characterization of certain diseases. Several neuroradiological signs have been
already described. The authors will present 15 neuroradiology signs in the present essay,
approaching their main characteristics, the significance of their role in the clinical practice,
as well as their respective imaging findings.
Keywords: Radiological signs; Neuroradiology; Computed tomography; Magnetic resonance
imaging.



INTRODUCTION
Descriptive terms in radiology are usually based on standards and consensus. There are
cases, however, in which the radiologist utilizes metaphors in the form of signs in allusion to
foods, animals or objects, to support his hypothesis for a given problem and describe the
findings of a particular disease. Signs, when present, are important as they allude to a more
specific diagnosis, and contribute with a certain degree of confidence in the diagnosis. In
general, "we recognize what we already know", and tools that aid in the interpretation of
images are valuable. In this first part, the authors discuss 15 neurological signs, with
illustrative images for each one of them.
"Ice-cream cone sign" of the temporal bone
The temporal bone ice-cream cone sign represents the normal appearance of the
malleoincudal joint on computed tomography (CT). The malleus (hammerhead) represents
the ice-cream ball, and the body of the incus (anvil) represents cone (Figure 1). The
anatomic identification of such structures is important, particularly in cases of trauma in
which ossicular luxation may occur.



"Cord sign" in cerebral venous thrombosis
Cerebral venous thrombosis (CVT) is a rare entity, with variable clinical presentations.
Seventy-five percent of the CVT occur in young women, between 20 and 40 years of age,
with the superior sagittal sinus (SSS) being most frequently affected (62% of cases). Such
increased incidence can be explained by pregnancy, puberty and use of oral
contraceptives
(1)
. The diagnosis can be achieved by means of CT (the most readily
available), magnetic resonance imaging (MRI) (the method of choice) or by conventional
angiography (CA) (the most invasive method). In 20% of cases, CT scans are normal. CVT
findings can be classified in direct and indirect. The cord sign and the empty delta sign are
direct signs of CVT. Indirect signs include: edema, infarction and hemorrhage. The cord sign
is characterized as increased density of the sinuses or of the cortical or deep veins (Figure
2), originated from the thrombosed material inside the affected vessel. The cord sign is
most frequently identified within two weeks after the first symptoms onset. With time, the
thrombus becomes isodense and subsequently, hypodense
(2)
.



"Empty delta sign" in venous sinuses thrombosis
The empty delta sign may occur in cases of CVT, characteristically involving the SSS. On
contrast-enhanced CT/MRI, the sign is characterized by a non-enhancing central triangular
shaped area (the thrombus itself), limited by enhancing dura mater (Figure 3)
(3)
. Numerous
factors may lead to CVT, as follows: inflammatory processes, infection, fibrosis of the
venous sinuses walls, direct tumoral compression or/and extension, and hypercoagulable
states
(4)
. The empty delta sign is usually not identified at the first week (the material is
isodense) as well as in chronic cases (more than two months), due to thrombus
recanalization
(5)
.



"Arrow sign" in ruptured middle cerebral artery aneurysm
In ruptured aneurysms the pattern of distribution of subarachnoid hemorrhage can indicate
its most likely location. In cases of bifurcation middle cerebral artery (MCA) aneurismal
rupture the bleed may present the shape of an arrow, with the shaft and the tip
representing blood in the horizontal segment of the Sylvian fissure and in the
frontotemporal opercular area, respectively (Figure 4)
(6)
.



"Dense artery sign" in acute middle cerebral artery infarction
The dense MCA sign is one of the early signs of infarct. This is due an increase in density of
its proximal segments, secondary to thrombosis (Figure 5). False-positive results may
occur, particularly in cases of parietal calcification. It is important to observe that the distal
branches of the MCAs rarely present parietal calcifications. Focal subarachnoid hemorrhage
may simulate an abnormally dense MCA especially when located at the Sylvian fissure and
constitute an additional cause for falsepositive results
(7)
.



"Dot sign" in acute middle cerebral artery infarction
The dot sign is one of the early signs of acute infarction and corresponds to a punctate
hyperdensity in the Sylvian fissure. The signal represents thrombosis in the M2 and M3
segments of the MCA on plain CT scans. The presence of a thrombus/clot within the vessel
alters and increases its density (Figure 6). The dot sign has a high specificity and high
positive predictive value, but has low sensitivity
(8)
.



"Hot nose sign" at brain death
The hot nose sign can be seen in cases of brain death and it is defined by the presence of
early and increased radiotracer activity in the nasopharyngeal region. It may also be seen
as an intense blush (hyperemia) at CA examinations (Figure 7). The phenomenon is a result
of a reduced blood flow in the internal carotid artery and increased flow in the external
carotid branches. Such signal is not exclusive of brain death and may be found in different
situations that lead to intracranial flow reduction in one or both internal carotid arteries
(9)
.



"Tau (t) sign" in persistent trigeminal artery
The sign of the Greek letter t occurs in cases of persistent trigeminal artery (PTA) and can
be identified at CA, CT angiography and MR angiography. PTA is the most prevalent type of
carotid-basilar anastomosis and it is formed by the horizontal and vertical segments of the
internal carotid artery (Figure 8). Despite being an incidental finding in the majority of the
cases, PTA is usually associated with basilar artery hypoplasia and can be accompanied by
oculomotor nerve palsy, trigeminal neuralgia or, eventually, with the presence of
aneurysms.
(10)

"Caput medusae sign" in developmental venous anomaly
The caput medusae sign is indicative of developmental venous anomaly (DVA), and is
identifiable at CA, CT and MRI. DVAs correspond to a network of dilated, abnormal
medullary veins with radial distribution, converging into a dominant, calibrous
transparenchymal vein, which may drain into a cortical vein, dural sinuses or into the deep
venous system (Figure 9). DVAs are the most frequent intracranial vascular abnormalities,
which are associated with cavernomas in around 30% of cases. Despite being considered
incidental findings, in some cases these may lead to intracranial hemorrhage, thrombosis
and venous infarction
(11)
. Hemorrhages secondary to DVA are rarely found, with an annual
risk of 0.7%
(12)
.



"Spoke wheel sign" in meningioma
The spoke wheel sign refers to the typical angiographic appearance found in meningiomas.
This sign corresponds to multiple small arteries radially distributed from a dominant feeding
artery (Figure 10). Meningiomas are the most common primary intracranial tumors in
adults. They are extra-axial, slow-growing, well-vascularized lesions with a benign behavior
(grade I, according to the World Health Organization). Another remarkable and very
common characteristic of meningiomas is the presence of a dural tail and, in 25% of cases,
hyperostosis of the adjacent bone
(13)
.



"Onion skin sign" in Bal's concentric sclerosis
The onion skin sign is considered pathognomonic for Bal's concentric sclerosis
(14)
.
According to the first reports on such disorder, most patients had an unfavorable history
with progression either to death or disability. Recent cases however, have presented a less
dramatic course. Bal's concentric sclerosis may occur as an isolated phenomenon or
precede the development of multiple sclerosis. The lesions present a peculiar pattern of
concentric lamellae of demyelination alternated with lamellae of myelinating or
remyelinating white matter. Such lesions are most frequently found in the frontal lobes, but
may be seen in the whole neuroaxis
(14)
. Magnetic resonance imaging (MRI) is the best
method for the disease diagnosis and follow-up. In spite of the high sensitivity of T2-
weighted images to demonstrate demyelinating lesions, the concentric rings are better
identified on T1-weighted images (Figure 11). The enhancement following contrast
administration is variable and probably represents active areas of demyelination
(15)
.



"Eccentric target sign" in toxoplasmosis
The eccentric or asymmetrical target sign is highly suggestive of central nervous system
toxoplasmosis. The sign represents a ring enhancing abscess associated with an enhancing
mural nodule (Figure 12). This finding is highly specific, but has low sensitivity, being found
in approximately 30% of cases. The pathological correlation of such sign is not completely
understood, but it is believed to represent internal folds and invaginations of the abscess
walls
(16)
.



"Reversal sign" in diffuse cerebral anoxia
Such sign is characterized by the relative inversion of attenuation between the supra and
infratentorial structures on unenhanced CT and may indicate diffuse brain ischemia. The
sign can explained by relative increase in the density of the cerebellum, basal ganglia and
thalami, and decreased density of the cerebral cortex and white matter (Figure 13).
Reversal sign can occur secondary to head trauma, hypoxia, birth anoxia, near drowning,
status epilepticus, hypothermia, bacterial meningitis and strangulation. The pathogenesis is
not completely clarified
(17)
.



"Dawson's fingers" in multiple sclerosis
The Dawson's finger's in multiple sclerosis are related to white matter inflammatory changes
that occur around the perimedullary veins. These are ovoid lesions, with the longest axis
perpendicular to the corpus callosum (Figure 14). James Walker Dawson was Scottish
pathologist who developed relevant studies on multiple sclerosis. The demyelinating plaques
are commonly located in the juxtacortical and periventricular white matter, in the corpus
callosum and callosal-septal interface, with high signal intensity on sequences with long
repetition time and hypo to iso-signal intensity on T1-weighted images
(18)
.



"Mount Fuji sign" in hypertensive pneumocephalus
This sign is seen in bilateral subdural hypertensive pneumocephalus. These air collections
cause compression of the frontal lobes, which take up a shape similar to the Mount Fuji
silhouette (Figure 15). Hypertensive pneumocephalus is a neurosurgical emergency, in
which the increased air pressure is thought to be secondary to a check-valve mechanism.
Air would enter freely into the subdural space by a defect in the bone but would not scape
with obstructive and the egress of air being blocked by an obstruction. This sign is useful in
the differentiation between hypertensive and non-hypertensive pneumocephalus.
Hypertensive pneumocephalus may after the drainage of subdural hematomas, following
skull base, paranasal sinuses and posterior fossa surgeries and in cases of head trauma
(19)
.



CONCLUSION
The aforementioned signs are important tools as they lead to a more specific diagnosis and
add a certain degree of confidence in the diagnosis. With such information, the assisting
physician can better establish the correlation between imaging and clinical findings.